B-Pah KO mice_Biocytogen Pharmaceuticals (Beijing) Co., Ltd._Biocytogen, drug development, antibody discovery

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B-Pah KO mice

Strain Name	C57BL/6N-^Pahtm1Bcgen/Bcgen	Common Name	B-Pah KO mice
Background	C57BL/6N	Catalog number	113306
Aliases	5053
NCBI Gene ID	PH, PKU, PKU1

Description

The PAH enzyme functions in liver as homo-tetramers to hydroxylate phenylalanine (Phe) into tyrosine and requires the co-factor tetrahydrobiopterin for catalysis. Phenylalanine hydroxylase (PAH) deficiency prevents the conversion of Phe to tyrosine. PAH deficiency results in hyperphenylalaninemia (HPA) and irreversible neurological consequences if left untreated.
The exons 1-13 of mouse Pah gene that encode the full-length protein were knocked out in B-Pah KO mice..
Pah protein expression was detected in the liver and kidneys of both female and male wild mice and heterozygous mice. Moreover, Pah protein expression of both female and male wild mice was higher than that of heterozygous mice.
B-Pah KO mice provide a powerful preclinical model for further development of gene and cell biologics to treat phenylketonuria(PKU).
Application: For example, this product is to add to the experimental tools available for PKU scientists and to advance PKU research.

Targeting strategy

Gene targeting strategy for B-Pah KO mice. The exons 1-13 of mouse Pah gene that encode the full-length protein were knocked out in B-Pah KO mice.

Protein expression analysis

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Western blot analysis of Pah protein expression in heterozygous B-Pah KO mice . Various tissue lysates were collected from wild-type C57BL/6N mice (+/+) and heterozygous B-Pah KO mice (H/+), and then analyzed by western blot with anti-Pah antibody(abcam, ab178430). 40 μg total proteins were loaded for western blotting analysis. Pah protein expression was detected in the liver and kidneys of both female and male wild mice and heterozygous mice. Moreover, Pah protein expression of both female and male wild mice was higher than that of heterozygous mice.